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Cardiac Wilson's disease.

P Kuan

    Chest
    |April 1, 1987
    PubMed
    Summary
    This summary is machine-generated.

    Cardiac issues are common in Wilson's disease, affecting 34% of patients with arrhythmias and autonomic dysfunction. This multisystem disorder requires adding cardiac evaluation to its known hepatic and neurological symptoms.

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    Area of Science:

    • Cardiology
    • Genetics
    • Internal Medicine

    Background:

    • Wilson's disease is a rare genetic multisystem disorder primarily affecting the liver and brain.
    • Cardiac involvement in Wilson's disease is infrequently recognized, despite its potential severity.

    Purpose of the Study:

    • To prospectively investigate the prevalence and spectrum of cardiac manifestations in patients with Wilson's disease.
    • To compare electrocardiographic findings between Wilson's disease patients and healthy controls.

    Main Methods:

    • Prospective study of 53 consecutive Wilson's disease patients and 40 control subjects (medical students and carriers).
    • Electrocardiography (ECG) performed on all participants.
    • Assessment of autonomic function using orthostatic hypotension tests and Valsalva maneuver.

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    Main Results:

    • Electrocardiographic abnormalities were observed in 34% of Wilson's disease patients, including hypertrophy and various arrhythmias.
    • Autonomic dysfunction, such as orthostatic hypotension, was present in 19% of patients.
    • Two cardiac deaths occurred, one due to ventricular fibrillation and the other to dilated cardiomyopathy.

    Conclusions:

    • Cardiac involvement is a significant, often unrecognized, feature of Wilson's disease.
    • Manifestations include arrhythmias, cardiomyopathy, cardiac death, and autonomic dysfunction.
    • Cardiac evaluation should be integrated into the comprehensive clinical assessment of Wilson's disease.